Literature DB >> 24875025

Outcomes and prognostic factors for Ewing-family tumors of the extremities.

Bivas Biswas1, Shishir Rastogi1, S A Khan1, B K Mohanti1, D N Sharma1, M C Sharma1, A R Mridha1, Sameer Bakhshi1.   

Abstract

BACKGROUND: There are few published studies describing the clinical results of patients uniformly treated for a Ewing-family tumor of an extremity.
METHODS: We performed a review of patients who had received uniform treatment consisting of neoadjuvant chemotherapy, surgery and/or radiation therapy as local treatment, and then adjuvant chemotherapy from June 2003 to November 2011 at a single institution.
RESULTS: There were 158 patients included in the study. The median age was fifteen years. Sixty-nine (44%) of the patients had metastatic disease at presentation. Fifty-seven patients underwent surgery, and forty-one received radical radiation therapy following neoadjuvant chemotherapy. After a median of 24.3 months (range, 1.6 to ninety-seven months) of follow-up, the five-year event-free survival, overall survival, and local control rates (and standard error) were 24.1% ± 4.3%, 43.5% ± 6%, and 55% ± 6.8%, respectively, for the entire cohort and 36.4% ± 6.2%, 57.6% ± 7.4%, and 58.2% ± 7.9%, respectively, for patients without metastases. In the multivariate analysis, metastases predicted inferior event-free survival (p = 0.02) and overall survival (p = 0.03) rates in the entire cohort, whereas radical radiation therapy predicted an inferior local control rate in the entire cohort (p = 0.001) and in patients without metastases (p = 0.04). In the group with localized disease, there was no difference between the patients who received radical radiation therapy and those who underwent surgery with regard to tumor diameter (p = 0.8) or post-neoadjuvant chemotherapy response (p = 0.1). A white blood cell count (WBC) of >11 × 109/L predicted inferior event-free survival (p = 0.005) and local control (p = 0.02) rates for patients without metastases.
CONCLUSIONS: To our knowledge, this is the largest study on extremity Ewing-family tumors treated with uniform chemotherapy and either surgical resection or radical radiation therapy in Asia. All possible efforts should be made to resect a primary tumor after neoadjuvant chemotherapy, as radical radiation therapy alone results in a poor local control rate despite a good post-neoadjuvant chemotherapy response. Patients without metastases but with a high WBC had inferior event-free survival and local control rates and may require more aggressive therapy. LEVEL OF EVIDENCE: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

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Year:  2014        PMID: 24875025     DOI: 10.2106/JBJS.M.00411

Source DB:  PubMed          Journal:  J Bone Joint Surg Am        ISSN: 0021-9355            Impact factor:   5.284


  11 in total

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2.  Identification of Patients With Localized Ewing Sarcoma at Higher Risk for Local Failure: A Report From the Children's Oncology Group.

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Review 7.  Management of Ewing sarcoma family of tumors: Current scenario and unmet need.

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8.  Does Radiotherapy after Surgery Affect Outcomes in Ewing's Sarcoma of the Pelvis?

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9.  Clinicopathological Features and Treatment Outcomes in Ewing's Sarcoma Patients: A 10-year experience of Alexandria Clinical Oncology Department.

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10.  Management of Ewing Sarcoma Family of Tumors: A Short Description of a Rare Primitive Uterine pPNET and Literature Review.

Authors:  Salvatore Pisconti; Giuseppina Della Vittoria Scarpati; Carlo Buonerba; Simona Messinese; Roberta Carella; Massimiliano Di Marzo; Giuseppe Di Lorenzo; Grazia Lazzari; Francesco Perri; Raffaele Solla
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